Polyglutamine Disorders (Paperback, Softcover reprint of the original 1st ed. 2018)

This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies' development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington's disease (HD); spinal-bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17). The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.

General

Imprint:	Springer International Publishing AG
Country of origin:	Switzerland
Series:	Advances in Experimental Medicine and Biology, 1049
Release date:	June 2019
First published:	2018
Editors:	Clevio Nobrega • Luis Pereira De Almeida
Dimensions:	235 x 155mm (L x W)
Format:	Paperback
Pages:	469
Edition:	Softcover reprint of the original 1st ed. 2018
ISBN-13:	978-3-319-89103-3
Categories:	Books > Medicine > Pre-clinical medicine: basic sciences > Medical genetics Books > Medicine > Clinical & internal medicine > Neurology & clinical neurophysiology Books > Science & Mathematics > Biology, life sciences > Life sciences: general issues > Neurosciences
LSN:	3-319-89103-0
Barcode:	9783319891033

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